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find Author "龚胜兰" 2 results
  • 以乳糜胸作为首发表现的原发性系统性淀粉样变性一例并文献复习

    目的探讨原发性系统性淀粉样变性的临床表现、诊断、治疗及预后,提高对累及胸膜的原发性系统性淀粉样变性的临床认识。方法报道四川大学华西医院收治的 1 例以乳糜胸作为首发表现的原发性系统性淀粉样变性患者,回顾性分析国内外文献。以“Amyloidosis,pleura”为检索词,在 PubMed 数据库检索,检索时间为 1968 年 12 月至 2019 年 9 月,共检测到相关英文文献 57 篇,报道 18 例患者。以“淀粉样变,胸膜”为检索词,在中国期刊网全文数据库检索,检索时间为 1981 年 3 月至 2019 年 9 月,共检索到相关中文文献 12 篇,报道 7 例患者。结果58 岁男性患者,临床表现为间断性背痛。查体:胸廓对称,双侧肩胛下角第 7 肋间各有一引流管,引流出乳白色液体。双下肺叩诊呈浊音,双下肺呼吸音低至消失,心脏及腹部查体未见异常。血清免疫固定电泳示可疑 IgA κ 型 M 蛋白条带,血清游离 κ 轻链检测 745 mg/L(参考值 6.7~22.4 mg/L)。胸部 CT 示双侧胸腔积液,双肺门淋巴结增大,腹部增强 CT 示胃壁广泛增厚伴腹腔、腹膜后广泛淋巴结增大,经多次胃镜行胃黏膜组织活检,胃组织刚果红染色阳性,胸腔积液流式细胞学见克隆性浆细胞,骨髓流式细胞学见 4% 克隆性浆细胞,确诊为原发性系统性淀粉样变性。在上述数据库中共检索 69 篇文献,经筛选除外资料不详后,有 18 篇文献 20 例患者明确诊断为胸膜淀粉样变性,符合检索条件。此类患者临床表现及影像学均无特异性,确诊依据胸膜活检刚果红染色阳性或胸腔积液查见淀粉样蛋白。结论原发性系统性淀粉样变性累及胸膜,临床表现及影像学缺乏特征性,与肿瘤胸膜转移、胸膜间皮瘤等疾病易混淆,但多数患者血清中会出现单克隆性免疫球蛋白增多,其诊断主要依靠活检组织病理学检查。治疗方案仍以化疗为主。

    Release date:2020-11-24 05:41 Export PDF Favorites Scan
  • A case of hypersensitivity pneumonitis presenting as progressive fibrotic interstitial lung disease and literature review

    ObjectiveTo strengthen the understanding to hypersensitivity pneumonitis and make early diagnosis and standard treatment by analyzing the clinical features, the diagnosis and treatment of a patient diagnosed as hypersensitivity pneumonitis presenting as progressive fibrotic interstitial lung disease in combination with literature review.MethodsThe diagnosis and treatment process and relevant clinical data of the patient were analyzed retrospectively and literatures were reviewed. Based on 282 relevant literatures, the diagnostic methods, treatment and prognostic factors of hypersensitivity pneumonitis were summarized.ResultsThe patient, female, 45 years old, self-employed, was admitted to the hospital due to "cough, sputum for 9 years, dyspnea for more than 6 months" without obvious extrapulmonary clinical manifestations. Creaks at the bottom of the lungs and clubbed toes were found through physical examination. High-resolution computed tomography indicated that the main manifestations were ground glass and grid-like shadows, presenting characteristics of interstitial pneumonia. The pulmonary lesions aggravated gradually, and part of the lung lobe presented honeycomb lung at the time of diagnosis. Eight years ago, she performed fibrobronchoscopy in other hospital revealing an increased lymphocyte proportion (39%) in bronchoalveolar lavage fluid. Lung function suggested very severe restrictive ventilation dysfunction. She was given prednisone for a short time, and the drug was stopped by herself with uncertain efficacy. Later, she performed frozen lung biopsy suggesting peribronchiolar metaplasia interstitial broadening with lymphocytic infiltration, without granuloma or fibroblast lesions. No obvious abnormity was seen in makers of infections and immunology. At this point, the patient was clearly diagnosed as hypersensitivity pneumonitis. Meanwhile, the patient's pulmonary lesions were still mainly made of ground glass and plaques, partly with changes like honeycomb. Poor response was obtained with anti-inflammatory treatment of prednisone for 3 months and anti-fibrosis treatment of pirfenidon for more than 2 months. Literature review resulted in 87 patients, including 39 males (44.82%) and 48 females (55.18%), with an average age of (47.0±18.4) years. Seventy patients (80.46%) had clear allergens. 3.45% patients' lymphocyte percentage in bronchoalveolar lavage fluid ranged from 20% to 39%, and 19.54% patients' lymphocyte percentage was more than 40%. 4.60% of the patients achieved remission through detachment from allergens; 71.27% of the patients achieved remission with glucocorticoid treatment, 14.94% improved with glucocorticoid treatment, and 1.15% died; 6.89% of the patients achieved remission after receiving anti-allergy therapy. Patients with chronic hypersensitivity pneumonia were found with poor prognosis.ConclusionsIn patients with diffuse interstitial pulmonary fibrosis with unknown cause, transbronchoscope freezing lung biopsy as early as possible is a feasible method for early diagnosis and improving prognosis. Patients with hypersensitivity pneumonitis with a long course of pulmonary fibrosis have a poor response to glucocorticoid and other classic treatments, and most of them have a poor prognosis.

    Release date:2021-04-25 10:17 Export PDF Favorites Scan
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